Advancements in the diagnosis and treatment of dermatofibrosarcoma protuberans
10.3760/cma.j.cn114453-20240604-00151-1
- VernacularTitle:隆突性皮肤纤维肉瘤的诊断与治疗现状
- Author:
Yangyang LU
1
;
Muran ZHOU
1
;
Aimei ZHONG
1
Author Information
1. 华中科技大学同济医学院附属协和医院整形外科，武汉 430022
- Publication Type:Journal Article
- Keywords:
Skin neoplasm;
Dermatofibrosarcoma protuberans;
Gene fusion;
Mohs surgery;
Imatinib
- From:
Chinese Journal of Plastic Surgery
2025;41(11):1200-1206
- CountryChina
- Language:Chinese
-
Abstract:
Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant cutaneous tumor characterized by a high propensity for local recurrence, yet low rates of metastasis and mortality. The pathogenesis of DFSP is primarily associated with a chromosomal translocation t(17; 22)(q22; q13), which leads to the fusion of the platelet-derived growth factor subunit B gene with the collagen type I alpha 1 chain gene in the majority of cases. Diagnosis relies on histopathological examination, supported by imaging studies for comprehensive assessment. Surgical resection remains the cornerstone of treatment; however, it is frequently complicated by a high risk of local recurrence. Recent advances in radiotherapy and targeted therapeutic strategies offer promising avenues for reducing recurrence rates and improving patient survival. Nevertheless, the pathological heterogeneity and diverse clinical manifestations of DFSP pose considerable challenges to the development and implementation of individualized treatment approaches. Thus, further fundamental research and refinement of clinical management are imperative to advance the diagnosis and therapy of DFSP.
